ESPE Yearbook of Paediatric Endocrinology

To read the full abstract: Endocr Rev. 2019;40(1):193–235

This paper combines a comprehensive review of current knowledge on pathophysiologic mechanisms contributing to hypothalamic obesity in craniopharyngioma patients with a systematic literature review of intervention studies for weight management in this patient population. Findings of both reviews are merged into evidence-based treatment algorithms for patients with hypothalamic obesity.

Hypothalamic obesity is a complex neuroendocrine disorder characterized by rapid weight gain, extreme obesity, hyperphagia, decreased resting energy expenditure, and reduced physical activity (1). In craniopharyngioma patients, tumor growth, neurosurgical procedures, and cranial irradiation damage key centers for the regulation of energy homeostasis located in the anterior (paraventricular nucleus), middle (arcuate nucleus, ventromedial nucleus), and posterior hypothalamus (dorsomedial nucleus, dorsal hypothalamic area). Altered eating behavior and extreme obesity develop in 40–50% of craniopharyngioma patients, and the most rapid, uncontrollable weight gain usually occurs during the first 6–12 months following neurosurgery (2). Although there are some promising reported results on the treatment of hypothalamic obesity (e.g. (3)), the overall effects on weight control are at best moderate, often heterogeneous, and have only been observed in small study populations.

As our understanding of the complex mechanisms controlling feeding behavior continue to improve, the authors of this timely review synthesize treatment paths for patients affected by hypothalamic obesity from available data of intervention studies. These treatment paths are strictly based on the underlying pathophysiology of hypothalamic obesity and cover six clinical domains, namely psychosocial problems, hyperphagia, sleeping problems, decreased energy expenditure, hyperinsulinemia, and hypopituitarism. This differentiated and evidence-based approach is not only of high value for those involved in the clinical care for this challenging patient population with highly specialized needs, but also represents a solid basis for the development of future clinical trials and state-of-the-art guideline recommendations.

References: 1. Bereket A, Kiess W, Lustig RH, Muller HL, Goldstone AP, Weiss R, et al. Hypothalamic obesity in children. Obesity Reviews. 2012;13(9):780–98.

2. Muller HL, Emser A, Faldum A, Bruhnken G, Etavard-Gorris N, Gebhardt U, et al. Longitudinal study on growth and body mass index before and after diagnosis of childhood craniopharyngioma. Journal of Clinical Endocrinology and Metabolism. 2004;89(7):3298–305.

3. Denzer C, Denzer F, Lennerz BS, Vollbach H, Lustig RH, Wabitsch M. Treatment of Hypothalamic Obesity with Dextroamphetamine: A Case Series. Obesity Facts. 2019;12(1):91–102.